Osteopetrosis caused by mutations of the IKBKG (inhibitor of nuclear factor kappa B kinase subunit gamma) gene, located on the X chromosome, occurs as a moderate complication of the OL-EDA-ID syndrome, lymphedema, anhidrotic ectodermal dysplasia and immunodeficiency (hence, the acronym) (62–65). This evidence concerns the gene IKBKG and ectodermal dysplasia syndrome.